Polycystic liver disease (PLD)

Polycystic liver disease (PLD)
Adult polycystic liver disease is an inherited disorder where many cysts of various sizes develop throughout your liver and is associated with congenital hepatic fibrosis.
Both conditions have in common a growth of numerous abnormally shaped (malformed) bile ducts, known as ductal plate malformation. PLD may occur with polycystic kidney disease (PKD) or alone but is less common and milder than the kidney form.

The tendency to form multiple cysts is probably present at birth, but cysts usually do not become larger and cause problems until you are an adult.

Cysts can be very small, perhaps no larger than a pinhead, but can grow up to 10cm. Similarly, your liver can remain its normal size or become heavily enlarged. Your liver should continue to function effectively in PLD and the disease is not considered to shorten your life expectancy.

PLD becomes more common with age but is rare, affecting much less than 1% of the population.

Women tend to be more affected as the size and increase of cysts is thought to be linked to levels of oestrogen.

Polycystic liver disease is fi rst noticed during puberty, with symptoms becoming more noticeable as you enter your thirties. Most people are diagnosed in their forties and fi fties. PLD is often without clear symptoms, but swollen, painful abdomen and an enlarged and hardened liver (hepatomegaly) are usually associated with the condition. In many cases polycystic liver disease is discovered by accident or noticed during diagnosis of kidney disease. The majority of people with polycystic kidney disease will also have PLD.

Laboratory tests such as liver and renal function tests may be used to diagnose PLD. Liver function tests (LFTs) are used to indicate whether your liver is inflamed (hepatitis), damaged or not working properly. They measure levels of certain enzyme and protein substances in your blood that may alter when liver damage is present. Doctors will also use imaging tests such as ultrasound, CT or MRI scan and a special X-ray of the kidney (intravenous pyelogram).

Cysts rarely require treatment. If necessary, laparoscopic de-roofing can be helpful for reducing larger, uncomfortable cysts. In rare cases of severe PLD, where multiple cysts cause the liver to become massively enlarged and very painful (leading to other complications), a liver transplant may be required. This is usually only recommended if your quality of life has become very poor and other treatments are no longer helpful.

The course of your treatment may be infl uenced by the degree of any associated kidney disease you may have.